Management of CML-blast crisis. Issue 3 (September 2016)

• Record Type: Journal Article
• Authors: Hehlmann, Rüdiger; Saußele, Susanne; Voskanyan, Astghik; Silver, Richard T.
• Is Part Of: Baillière's best practice and research in clinical haematology. Volume 29:Issue 3(2016)
• Page Start: 295
• Page End: 307
• Publication Date: 2016-09
• Subjects: CML blast crisis -- Diagnosis -- Therapy -- Early prediction -- Prevention; Hematology -- Periodicals; Blood -- Periodicals; Hematologic Diseases -- Periodicals; Electronic journals; 616
• DOI: 10.1016/j.beha.2016.10.005
• Languages: English
• ISSNs: 1521-6926

Abstract

Abstract: Tyrosine kinase inhibitors (TKI) have moderately improved survival in BC, but a median survival of less than 1 year is still unsatisfactory. This article reviews the various tests required for diagnosis of BC, features at diagnosis, treatment modalities (intensive chemotherapy, TKI, allo-SCT and a selection of investigational agents), options of prevention and predictors of progression. The best prognosis is observed in patients that achieve a 2nd CP. Allo-SCT probably further improves prognosis of patients in 2nd CP. The choice of TKI should be directed by the mutation profile of the patient. BC can be prevented. A careful analysis of risk factors for progression may help. Current treatment options are combined in a concluding strategy for the management of BC. Highlights: BCR-ABL is the driving force of progression. Clonal evolution indicates progression. BC patients who achieve a 2nd CP have the best prognosis. Allo-SCT should be tried in 2nd CP whenever a donor is available and the patient can tolerate the procedure. BC can be prevented by elimination (or reduction) of BCR-ABL. Much is known on genetic instability and clonal evolution as causes of BC, but confirmation of our understanding by successful intervention as proof of principle is lacking.